Intestines amyloidosis
Contents:
Description:
Intestines amyloidosis - an intestines disease (an independent disease or "the second disease") caused by adjournment in its fabrics of amyloid.
At an amyloidosis all digestive tract can be surprised, but the most considerable adjournment of amyloid happens in a small intestine.
Intestines amyloidosis reasons:
Development of an amyloidosis is connected with disturbance of proteinaceous and synthetic function of reticuloendothelial system, accumulation in a blood plasma of the abnormal proteins serving as autoantigens and causing formation of autoantibodies. Interaction of antigen with an antibody is resulted by sedimentation of the coarse-dispersion proteins participating in formation of amyloid. Being postponed in fabrics (for example, in walls of vessels, ferruterous, etc.), amyloid forces out functionally specialized elements of body that leads to death of this body.
Pathogeny:
AL amyloidosis (immunoglobulin light chains derived) - primary amyloidosis caused by accumulation in a blood plasma of abnormal light chains of the immunoglobulins synthesized by malignizirovanny V-lymphocytes at a multiple myeloma (Rustitsky-Kalera's disease)
AA amyloidosis (acquired) - the secondary amyloidosis caused by hypersecretion a liver of protein of an acute phase of alpha globulin in response to chronic (is more often purulent) an inflammation. The leprosy, a bronchoectatic disease, tuberculosis etc. is an example of such inflammations. Subspecies of this amyloidosis is the ASC amyloidosis (systemic cardiovascular) developing at persons over 90 years. The reasons of a pathogeny are unknown.
AF amyloidosis (the Mediterranean alternating fever) - a hereditary form of an amyloidosis, with the autosomal and recessive mechanism of transfer. The people belonging to the certain ethnic groups living on the coast of the Mediterranean Sea (Sephardic Jews, Greeks, Arabs, Armenians) suffer from this type of an amyloidosis. There are kinds of an amyloidosis characteristic of a certain geographical area: "the Portuguese amyloidosis" (with preferential damage of nerves of the lower extremities), "the American amyloidosis" (with preferential damage of nerves of upper extremities), a family nefropatichesky amyloidosis, or the "English amyloidosis" proceeding with symptoms of a small tortoiseshell, deafness and fever.
The AH amyloidosis (hemodialisis-related) - is observed only at the patients who are on hemodialysis treatment. The pathogeny is connected with the fact that, normal utilized kidneys beta 2 - class MHC I microglobulin, is not filtered in the haemo dialyzer and collects in an organism.
AE amyloidosis - the form of a local amyloidosis developing in some tumors, for example, in medullary cancer of C-cells of a thyroid gland. In this case a predecessor of amyloid are pathological fragments of a calcitonin.
Amyloidosis of the Finnish type - the rare type of an amyloidosis caused by a mutation of a gene of GSN coding protein джелсолин.
Tissue specimen
Intestines amyloidosis symptoms:
The amyloidosis of intestines is shown by a sensation of discomfort, weights, more rare moderate stupid or spastic abdominal pains, disturbances of a chair: locks or persistent ponosa. In blood anemia, a leukocytosis, increase in SOE, a hypoproteinemia (at the expense of a hypoalbuminemia), a hyperglobulinemia, a hyponatremia, a prothrombinopenia, a hypocalcemia.
Complications: a heavy hypoproteinemia owing to disturbance of processes of absorption in intestines, polyhypovitaminoses, an intestines stenozirovaniye, amyloid ulcers, intestinal bleedings, perforation.
Diagnosis:
The following signs can help with diagnosis of an amilovdoz of intestines:
Existence of a basic disease which leads to development of an amyloidosis of intestines (tuberculosis, a bronchoectatic disease, a pseudorheumatism, etc.).
Persistent diarrhea, resistant to therapy by the antibacterial, knitting, adsorbing and fixing means (an amyloidosis with preferential damage of a small intestine).
Clinical picture of a sprue (it is characteristic of an amyloidosis with preferential defeat of a small bowel).
Treatment of an amyloidosis of intestines:
General principles of treatment of an amyloidosis:
The mode is house, except for serious conditions (the expressed heart failure, a chronic renal failure).
The sick amyloidosis showed long (1,5-2 years) reception of a crude liver (on 100-120 g/days).
Restriction of consumption of protein, salt to patients with a chronic renal failure.
Salt restriction to patients with heart failure.
At a secondary amyloidosis - treatment of a basic disease (tuberculosis, osteomyelitis, a pleura empyema, etc.) after which treatment also manifestations of an amyloidosis quite often disappear.
Transfer of the patient with a dialysis amyloidosis on peritoneal dialysis
At the intestines amyloidosis proceeding with persistent diarrhea - astringents (bismuth subnitrate, adsorbents).
At primary amyloidosis in initial stages of process - chloroquine of 0,25 g 1 р / days it is long, a combination of Melphalanum and Prednisolonum, Melphalanum, Prednisolonum and colchicine or only colchicine.
At a secondary amyloidosis - specific treatment of a basic disease
At a family amyloidosis - colchicine (on 0,6 mg 2-3 р / days).
Symptomatic therapy: the vitamins, diuretic means, drugs reducing pressure, plasma transfusion, etc.
Surgical treatment of an amyloidosis:
Removal of a spleen can improve a state owing to reduction of amount of the amyloid which is formed in an organism.
